Hum Genet (1984) 68:148-153
© Springer-Verlag 1984
The identification of a DNA polymorphism of the a fibrinogen gene,
and the regional assignment of the human fibrinogen genes to 4q26-qter
S. E. Humphries 1 , A. M. A. Imam 3 , T. P. Robbins I, M. Cook 1 , B. Carritt 2, C. Ingle 1 , and R. Williamson 1
1 Department of Biochemistry, St. Mary's Hospital Medical School, Paddington, London W2 1PG
~MRC Human Biochemical Genetics Unit, University College, London, Great Britain
Summary. We have identified a common restriction fragment
length polymorphism of the c~ fibrinogen gene with the enzyme
TaqI. This polymorphism is probably due to a single base
change that creates or destroys a TaqI recognition site about
1000 basepairs from the 3' end of the c~ fibrinogen g~ne. The
frequency of the rare allele in 83 unrelated healthy individuals
is 0.33. We have used in situ hybridisation of the a fibrinogen
cDNA to localise the gene on chromosome 4@9-31. We have
confirmed this regional localisation by restriction fragment
detection in a human × Chinese hamster somatic cell hybrid
which contains a translocated human chromosome 4 with a
breakpoint at 4q26. The a,/3, and 3' fibrinogen genes are all
present on human chromosome 4q26-qter.
Introduction
Fibrinogen (factor I) is an abundant plasma protein which par-
ticipates in the final steps of the coagulation pathway in higher
vertebrates. A number of genetically determined variants for
human fibrinogen have been reported (Bloom 1981) many of
which have an increased clotting time, some with pathological
consequences. Elevated levels of fibrinogen and other coagu-
lation factors have also been correlated with a predisposition
to ischaemic heart disease (Mead and North 1977; Mead et al.
1980).
Native fibrinogen (tool. wt. 340,000) is a symmetrical tri-
nodular molecule composed of dimers of c~,/3, and 3" chains
linked by disulphide bridges. The complete amino acid
sequences of all three chains have been determined (Blom-
back et al. 1976; Lottspeich and Henschen 1977; Doolittle et
al. 1979; Watt et al. 1979). The chains show sequence homo-
logy suggesting that a common ancesteral sequence under-
went duplication and divergence. This relationship has been
confirmed recently with the isolation and sequencing of
cDNA clones coding for rat and human fibrinogen chains
(Crabtree and Kant 1981; Kant et al. 1983a; Rixon et al. 1983;
Chung et al. 1983a,b; Imam et al. 1983).
The gene for 3"fibrinogen has been shown to be on the long
arm of human chromosome 4 by linkage of a 3' chain variant
to the MNSs blood group locus (Olaisen et al. 1982). It has
recently been demonstrated that in both humans and rats, the
fibrinogen genes are physically linked (Kant and Crabtree
3 Present address: Institute for Cancer Research, Fulham Road,
London SW3, England
Offprint requests to: S. E. Humphries
1983; Kant et al. 1983b) and it would therefore seem likely
that in the human genome all three fibrinogen genes are clus-
tered on the long arm of chromosome 4 (Kant et al. 1983b).
We have confirmed this by in situ hybridisation of the a fibri-
nogen cDNA clone and by Southern blot hybridisation to
DNA from a hybrid cell line with a translocation involving
4q26-qter. We also report a common restriction fragment
length polymorphism (RFLP) of the c~ fibrinogen gene that
will be useful for linkage studies and mapping the chromo-
some.
Materials and methods
Gene probes for the fibrinogen chain
The c~ and 3' fibrinogen probes (designated pAF1 and pGF1
respectively) have been described previously (Imam et al.
1983). They consist of inserts of 1950bp (pAF1) and 950bp
(pGF1) in the PstI site of the vector pKT218, and contain the
majority of the coding region and probably all of the 3'
untranslated region. The fi fibrinogen probe (pBF5) consists
of an insert of 1450bp in pKT218. A partial sequence of
this clone is presented in Fig. 1. This confirms that the clone
codes for/3 fibrinogen, and from restriction fragment mapping
(A. Imam unpublished work) it is likely that the clone contains
the majority of the coding region and probably all of the 3'
untranslated region. Plasmid DNA was prepared by a modi-
fied cleared lysis method (Birnboim and Doly 1979) and purif-
ed on CsC1 gradients.
277
Ala Thr Asn Thr Asp Gly Lys
CTGCAGGGGG GGGGGGGGGG GGGGGGGGGG T GCA ACC AAC ACA GAT GGG AAG
i0 20 30 40 50
PstI
287 297
Asn Tyr Cys Gly Leu Pro Gly Glu Tyr Trp Leu Gly Asn Asp Lys
AAT TAC TGT GGC CTA CCA GGT GAA TAT TGG CTT GGAAAT GAT AAA
60 70 80 90
307
Ile Ser Gln Leu Thr Arg Met Gly Pro Thr Glu Leu Leu Ile Glu
ATT AGC CAG CTT ACC AGG ATG GGA CCC ACA GAA CTT TTG ATA GAA
zoo zlo 12o z3o z4o
Fig. 1. Partial nucleotide sequences of the clone pBFI and the corre-
sponding amino acid sequences. The numbering of amino acids is as
reported in the literature. The recombinant was sequenced by sub-
cloning PstI fragments into the PstI site of the M13 rap9 poly linker,
as previously described (Imam et al. 1983)